Autoimmune and Paraneoplastic Neurological Disorders

Autoimmune neurology is a rapidly emerging subspecialty that encompasses the diagnosis and treatment of neurological disorders with an autoimmune (paraneoplastic or non-cancer associated) basis. There has been a dramatic rise in discoveries of neural-specific autoantibodies and their target antigens. Laboratory testing is now available for most of these neural-specific autoantibodies, which serve as diagnostic markers directing the physician towards specific cancer types and assisting in therapeutic decision making. Autoimmune neurology intersects with many traditional neurological subspecialties including cognitive behavioral neurology, movement disorders, epilepsy, neuro-oncology, neuromuscular disorders, autonomic neurology, and demyelinating disorders. Antibodies targeting intracellular proteins (nuclear and intracytoplasmic enzymes, transcription factors, RNA binding proteins) serve as markers of neural peptide-specific cytotoxic effector T-cell mediated injury and are generally poorly responsive to immunotherapy. In contrast, antibodies targeting plasma membrane proteins (neurotransmitter receptors, ion channels, water channels, channel complex proteins) may act as pathogenic effectors and imply immunotherapy responsiveness.